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New model of neurodegenerative disease

14 February 2014

 3 stages of FUS (green) aggregation in a mammalian cell

3 stages of FUS (green) aggregation in a mammalian cell 

A team of researchers from the School of Biosciences led by Dr N Ninkina and Prof V Buchman have established a new experimental model for one of the most common hereditary forms of Motor Neuron Disease, Amyotrophic Lateral Sclerosis (ALS).  This work has recently been selected as one of the best research studies published in The Journal of Biological Chemistry in 2013.

This subtype of a devastating neurodegenerative disease, is thought to occur following the accumulation of a protein called FUS in particular neurones of affected patients, resulting in severe neurodegeneration and neuro-muscular problems.  The research group’s findings reveal that FUS aggregation within neurons is sufficient to cause an ALS-like pathology similar to that observed in ALS patients.

“Such models of human disease are vital for understanding the chain of events that lead to the development diseases, and are central to facilitate the design of novel strategies for therapeutic intervention in ALS and related neurodegenerative disorders.” Dr Ninkina explains. “Our model is quite unique because it reproduces many important features of human ALS with high reproducibility and within a convenient timeframe.”

This study was carried out in collaboration with researchers from three Russian research organisations (Institute of Physiologically Active Compounds Russian Academy of Sciences, Institute of Gene Biology Russian Academy of Sciences and Pirogov Russian National Research Medical University), whilst valuable tools were received from colleagues in the University of California at San Diego. The work was funded in part by the Wellcome Trust and by several Russian Research Grants. Recently the group have been awarded a Project Grant by the Motor Neurone Disease Association (to start in April 2014) to continue and extend their studies of FUS-triggered ALS.